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学科主题: 生命有机化学
题名: EF-hand domains of MCFD2 mediate interactions with both LMAN1 and coagulation factor V or VIII
其他题名: EF-hand domains of MCFD2 mediate interactions with both LMAN1 and coagulation factor V or VIII
作者: ZHENG CHUNLEI ; LIU HUIHUI ; Zhou JH(周佳海) ; ZHANG BIN
刊名: Blood
发表日期: 2010
卷: 115, 期:5, 页:1081-1087
收录类别: SCI
部门归属: 美国Cleveland Clin Fdn; 中国科学院上海有机化学研究所
英文摘要: Combined deficiency of factor V and factor VIII (F5F8D) is a bleeding disorder caused by mutations in either LMAN1 or MCFD2. LMAN1 (ERGIC-53) and MCFD2 form a Ca2+-dependent cargo receptor that cycles between the endoplasmic reticulum (ER) and the ER-Golgi intermediate compartment for efficient transport of FV/FVIII from the ER to the Golgi. Here we show that the C-terminal EF-hand domains are both necessary and sufficient for MCFD2 to interact with LMAN1. MCFD2 with a deletion of the entire N-terminal non-EF hand region still retains the LMAN1-binding function. Deletions that disrupt core structure of the EF-hand domains abolish LMAN1 binding. Circular dichroism spectroscopy studies on missense mutations localized to different structural elements of the EF-hand domains suggest that Ca2+-induced folding is important for LMAN1 interaction. The EF-hand domains also mediate the interaction with FV and FVIII. However, mutations in MCFD2 that disrupt the tertiary structure and abolish LMAN1 binding still retain the FV/FVIII binding activities, suggesting that this interaction is independent of Ca2+-induced folding of the protein. Our results suggest that the EF-hand domains of MCFD2 contain separate binding sites for LMAN1 and FV/FVIII that are essential for cargo receptor formation and cargo loading in the ER. (Blood. 2010;115:1081-1087)
语种: 英语
相关网址: 查看原文
WOS记录号: WOS:000274287500022
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内容类型: 期刊论文
URI标识: http://ir.sioc.ac.cn/handle/331003/27801
Appears in Collections:上海有机化学研究所_期刊论文

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Recommended Citation:
ZHENG CHUNLEI,LIU HUIHUI,Zhou JH,et al. EF-hand domains of MCFD2 mediate interactions with both LMAN1 and coagulation factor V or VIII[J]. Blood,2010,115(5):1081-1087.
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